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DLBCL : Central Nervous System

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Comments:
Diffuse large B-cell lymphomas (DLBCL) make up the bulk of primary central nervous system lymphomas (PCNSL), especially in immunocompromised patients (HIV infection, transplant recipients, congenital immunodeficiency disorders). The immunocompromised patients tend to be younger with a striking male predominance. Immunocompetent patients tend to be older (median age 6th decade).

DLBCLs express pan B-cell markers such as CD20, CD22, CD79a, and PAX5, and monotypic immunoglobulin (usually IgM). HIV-associated PCNSL commonly express BCL2, LMP-1, EBV nuclear antigen, and EBV-coded small RNA (EBER) by in-situ hybridization. Immunoglobulin genes are clonally rearranged.

This autopsy specimen shows large hemorrhagic and necrotic lesions involving the white matter of bilateral parietal lobes in a HIV+ve patient. The morphologic and immunophenotypic findings on biopsy were diagnostic of diffuse large B-cell lymphoma. The patient succumbed to the tumor within 6 months after presentation despite chemotherapy and radiation.

Additional images of primary CNS lymphomas are here

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